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References20
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Published on Dec 1, 2018
Giampaolo Merlini77
Estimated H-index: 77
(UNIPV: University of Pavia),
Angela Dispenzieri96
Estimated H-index: 96
(Mayo Clinic)
+ 4 AuthorsMorie A. Gertz100
Estimated H-index: 100
(Mayo Clinic)
Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. The disease is progressive and, accordingly, early diagnosis is vital to prevent irreversible organ damage, of which cardiac damage and renal damage predominate. The development of novel sensitive biomarkers and imaging technologies for the det...
Published on Aug 1, 2018in Leukemia9.94
Arjun Lakshman4
Estimated H-index: 4
(Mayo Clinic),
Shilpa Paul1
Estimated H-index: 1
(Mayo Clinic)
+ 23 AuthorsDavid Dingli54
Estimated H-index: 54
(Mayo Clinic)
Published on Nov 1, 2017in British Journal of Haematology5.21
Sajitha Sachchithanantham10
Estimated H-index: 10
(UCL: University College London),
Oscar Berlanga1
Estimated H-index: 1
+ 6 AuthorsAshutosh D. Wechalekar36
Estimated H-index: 36
(UCL: University College London)
Summary Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. HLC measurements identified immunoparesis of at least one immunoglo...
Published on Oct 2, 2017in Amyloid4.92
Luis Gerardo Rodríguez-Lobato2
Estimated H-index: 2
(University of Barcelona),
C. Fernández de Larrea15
Estimated H-index: 15
(University of Barcelona)
+ 8 AuthorsJordi Yagüe36
Estimated H-index: 36
(University of Barcelona)
AbstractObjectives: Immunoparesis (IP) is a risk factor associated with an unfavourable outcome in several plasma cell disorders. It has been suggested that its presence in light-chain (AL) amyloidosis could be associated with worse prognosis. However, the relevance of IP after treatment has not been evaluated to date. The aim of this study was to determine the prognostic impact of IP at diagnosis and one year after treatment onset in patients with AL amyloidosis.Methods: The clinical records of...
Published on Jul 1, 2017in Leukemia9.94
Eli Muchtar10
Estimated H-index: 10
(Mayo Clinic),
Angela Dispenzieri96
Estimated H-index: 96
+ 19 AuthorsNelson Leung51
Estimated H-index: 51
Interphase fluorescence in situ hybridization in untreated AL amyloidosis has an independent prognostic impact by abnormality type and treatment category
Published on Apr 13, 2017in Blood16.56
Eli Muchtar9
Estimated H-index: 9
,
Morie A. Gertz100
Estimated H-index: 100
+ 21 AuthorsNelson Leung51
Estimated H-index: 51
(Mayo Clinic)
In light of major advances in immunoglobulin light chain (AL) amyloidosis, we evaluated the trends in presentation, management, and outcome among 1551 newly diagnosed AL amyloidosis patients seen in our institution from 2000 to 2014. As compared with the 2 intervals 2000-2004 and 2005-2009, patients diagnosed in 2010-2014 were less likely to have >2 involved organs. Utilization of autologous stem cell transplant (ASCT) was similar across all periods, about one-third of patients, but there was an...
Published on Jan 1, 2017in Leukemia9.94
Eli Muchtar10
Estimated H-index: 10
(Mayo Clinic),
Angela Dispenzieri96
Estimated H-index: 96
+ 17 AuthorsWilson I. Gonsalves19
Estimated H-index: 19
Immunoparesis is an adverse prognostic marker in plasma cell proliferative disorders. Its impact in AL amyloidosis has not been explored in depth. Newly diagnosed AL amyloidosis patients (n=998) were evaluated for immunoparesis by two methods. The first method was qualitative, considering the number of suppressed uninvolved immunoglobulins below the lower limit of normal (LLN) (none, partial, all). The second method was quantitative, assessing the average relative difference (ARD) of the uninvol...
Published on Sep 1, 2016in Haematologica7.57
Eli Muchtar10
Estimated H-index: 10
(Mayo Clinic),
Angela Dispenzieri96
Estimated H-index: 96
(Mayo Clinic)
+ 15 AuthorsRajshekhar Chakraborty7
Estimated H-index: 7
(St. Joseph Hospital)
Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglobulins, classifying patients into a negative average relative difference (i.e. significant immunoparesi...
Published on Jan 1, 2016in Acta Haematologica1.31
Eli Muchtar10
Estimated H-index: 10
(Mayo Clinic),
Francis Buadi47
Estimated H-index: 47
(Mayo Clinic)
+ 1 AuthorsMorie A. Gertz100
Estimated H-index: 100
Immunoglobulin amyloid light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, where the culprit amyloidogenic protein is immunoglobulin light chains produced by marrow clonal plasma cells. AL amyloidosis is an infrequent disease, and since presentation is variable and often nonspecific, diagnosis is often delayed. This results in cumulative organ damage and has a negative prognostic effect. AL amyloidosis can also be challenging on the diagnostic level, especially when dem...
Published on May 1, 2015in Blood Cancer Journal7.89
Rahma Warsame10
Estimated H-index: 10
(Mayo Clinic),
S Kumar35
Estimated H-index: 35
+ 16 AuthorsRhett P. Ketterling55
Estimated H-index: 55
Abnormal FISH in patients with immunoglobulin light chain amyloidosis is a risk factor for cardiac involvement and for death
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